Von Recklinghausen's disease is characterized by multiple neurofibromas, pigmentations, and pachydermatoceles of the skin depending on the disorder of the neural crest derivative. We report on a 25-year-old patient suffering from neurofibromatosis localized in the cranio-orbital region, whose main problem was a pulsating right exophthalmos caused by herniation of the frontotemporal lobe through a defect of the greater wing of the sphenoid. The lesion was approached through a combined route (i.e., transfrontally and transfacially [upper eyelid incision]). Surgical strategy and associated problems are discussed.